It is a rare condition that involves inflammation of the lining of large blood vessels called arteries.
In 1830, Rokushu Yamamoto published the first description of Takayasu’s disease.
This inflammation can seriously damage major arteries, including those that supply blood to the heart and lungs. Takayasu arteritis is sometimes called aortic arch syndrome .
Takayasu arteritis belongs to a family of arthritic diseases called vasculitis. The word vasculitis means inflammation of the blood vessels.
As an autoimmune disease, Takayasu arteritis occurs when the body’s immune system attacks its own healthy cells and tissues. The reason it does this is not well understood.
It is very important to treat Takayasu’s arteritis quickly and aggressively to control the inflammation that can damage the arteries.
If left untreated, it can lead to serious complications, such as vision loss and strokes.
Takayasu arteritis mainly affects young people, mainly between the ages of 10 and 40 years.
It is 8 times more common in young women than young men, and slightly more common in people of Asian or African descent.
Some people with Takayasu’s arteritis can go into remission after successful treatment and eventually no longer require medication.
In others, it behaves more like a chronic disease that must be controlled with medication on an ongoing basis.
Causes of Takayasu’s arteritis
The underlying cause of Takayasu’s arteritis is unclear and is suspected to be related to immune medications.
It shares many similarities with giant cell arteritis that occurs predominantly in people over the age of 60.
Symptoms of Takayasu’s arteritis
The early signs and symptoms of Takayasu’s arteritis are often difficult to identify.
People with the condition generally begin to feel fatigued and generally feel generally unwell.
Many patients with Takayasu’s arteritis think they have a viral illness such as a cold or the flu.
Symptoms may include: fever, headache, and muscles that may feel sore.
Loss of appetite and unintentional weight loss can also occur.
As Takayasu’s arteritis progresses, the blood vessels become inflamed and tender.
This is especially common in the carotid arteries in the neck, the blood vessels that supply blood to the brain, which are close to the surface of the skin.
Depending on which arteries are affected, the symptoms of Takayasu’s arteritis could be different:
Heart or aorta
If the blood vessels of the heart are involved, patients may feel chest pain.
Eventually, the inflammation can cause swelling of the blood vessel walls and blockage of the arteries.
If the aorta, the body’s main artery, is affected, the heart may have trouble pumping blood properly.
Carotid (neck) arteries
A stroke or seizure can occur. The carotid arteries supply blood to the brain.
Vertebral arteries (vessels that supply blood to the head)
Dizziness and visual problems can occur.
Extremities (arms or legs)
A person may lose a pulse in the limb, feel weak, or notice a color change in the affected limb.
If the arteries near the kidney are affected, your blood pressure may be high.
Diagnosis of Takayasu arteritis
The diagnosis is suspected based on the history and then confirmatory tests are ordered to establish the diagnosis.
The doctor will perform a complete exam that includes the chest, extremities, abdomen, and a neurological exam. Patients can often have high blood pressure and / or abnormal pulses.
Inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein may be elevated in active disease.
The definitive diagnosis of Takayasu arteritis is made by looking at images of the affected blood vessels. Commonly performed tests are:
- MRA- Magnetic Resonance Angiography: This involves the introduction of a contrast dye into the vessels to help visualize them.
- CTA – Computed Tomography Angiography: This also involves inserting a dye but uses a CT machine to obtain images.
- Color Doppler Ultrasound: This is the use of ultrasound waves to image the vessels and can also detect the flow of blood through them.
Obtaining tissue from a major vessel is difficult and therefore rarely done, but can be done to confirm disease activity if possible.
Complications of Takayasu arteritis
Arterial stenosis is a narrowing of the blood vessels that results in a poor blood supply to the organs. It can affect the renal vessels such as renal artery stenosis.
An aneurysm is an abnormal dilation of the vessel. Ongoing inflammation can weaken the vessel walls leading to balloon dilation.
This can affect the downstream blood supply and also poses a risk of rupture.
Regurgitation of the aortic valve
When the disease affects the early part of the aorta, it can also damage the aortic valve and cause a leak of blood around the valve causing aortic regurgitation.
The involvement of the arteries that supply the brain can cause damage that can lead to a stroke
Takayasu arteritis treatment
The goal of treating Takayasu arteritis is to control the ongoing inflammation. The following medications are generally used:
Steroids or glucocorticoids such as prednisone
This is the treatment of choice and is generally effective in controlling the disease and produces symptomatic improvement.
In case of long-term need for steroids or lack of response to steroids, alternative medications are also effective, such as methotrexate, leflunomide, azathioprine, and mycophenolate mofetil.
These are newer groups of drugs that are antibodies directed against substances in the body that lead to inflammation. Tocilizumab, infliximab, and etanercept are drugs that are being investigated for use in this condition.
For complications of Takayasu arteritis, such as strictures and aneurysms, surgical treatment options can be considered.
It can be done through a minimally invasive route by inserting a lead through a peripheral vessel to reach the affected area to open narrowed vessels.
This is a surgical procedure that is preferred when large portions of the vessel are affected and involves replacing the vessel tract with a graft.
Aortic valve surgery
In the case of aortic regurgitation, the aortic valve can be repaired or replaced as appropriate.
Takayasu arteritis prognosis
This is a chronic condition that undergoes waves of disease exacerbation and resolution.
It can be controlled effectively with the use of steroids alone, although the side effects can be troublesome.
In many people, the disease can resolve completely, leading to discontinuation of therapy. However, it is important to monitor the return of symptoms in these cases.